Evolución del desarrollo neurosensorial de los recién nacidos de muy bajo peso (≤ 1.500 g) en nuestro medio / Neurosensorial developmental follow-up of very low weight newborns (<1.500g) in our center

Objetivos: Estudiar la situación y evolución neurológica de los recién nacidos de muy bajo peso (≤ 1.500 gramos) en nuestro medio. Material y métodos: Estudio retrospectivo sobre la evolución neurológica de 268 recién nacidos de muy bajo peso (RNNB) durante un período de seguimiento de 5 años, a través de visitas periódicas a cargo de neonatólogos, neuropediatra y psicólogo y colaboración de otro servicios, realizándose exploraciones oftalmológicas, EEG y potenciales evocados. Período de estudio: 1993-1999. Se realizó estudio estadístico de un amplio número de variables con el programa SPSS para Windows. Las secuelas se clasificaron según la gravedad en leves, moderadas, graves, y en relación al tipo de deficiencia, en motoras, cognitivas, sensoriales, mixtas y plurideficiencias (cuando se asociaban una o más secuelas). Se analizaron también a frecuencia y mortalidad de los RN;BP a lo largo de los años de estudio y siguientes. Resultados: Resaltaba una media de peso baja para la edad gestacional (EG), edad materna superior a 30 años, elevado porcentaje de recién nacidos de bajos peso, cesáreas, patología obstétrica, complicaciones neurológicas y extraneurológicas, tratamientos médicos y ventilación mecánica (VM), porcentaje bajo de gestantes tratadas con corticoides y/o tocolíticos y de prematuros con surfactante. Reproducción asistida: 17,68%. Número de fetos > 1 el 37%. La tasa de mortalidad neonatal de los TNMBP ha ido disminuyendo en los últimos años, siendo de 17,50% en el 2005, 14,45% en el 2006 y 11,18% en el 2007. Asimismo, su incidencia ha ido incrementándose, siendo el 19% en el 2005, 18,2% en el 2006 y 21,8% en el 2007. Presentaron secuelas el 30% con tendencia ascendente, leves 46,5%, moderadas, 22,1% graves, 31,4%, predominando las motoras puras (43%), seguidas por las mixtas (27,9%), y plurideficiencias (17,4%). Hipoacusia neurosensorial, 4,8%. Retinopatía del prematuro (ROP), 17,2%. Parálisis cerebral (PC), 17,16%. El 40% tenían algún tipo de alteración en la ecografía transfontanelar (ECO), hemorragia intra/periventricular (HPIV), 22,7%, leucomalacia periventricular (LPV), 15,3%, trastornos del aprendizaje, 33% de los escolarizados. Tuvieron alta correlación estadística con la aparición de secuelas la enfermedad de la membrana hialina (EMH) displasia broncopulmonar (DBP), sepsis tardía, necesidad de oxígeno (O2) y ventilación mecánica (VM) y muy especialmente, la edad gestacional (EG) y alteraciones de la ecografía craneal. Conclusiones: Se observa incremento en la frecuencia y supervivencia de los RNMBP y una incidencia de secuelas similar a otras series con un alto porcentaje de graves. Las secuelas neurológicas se relacionan principalmente con la EG y la patología especialmente del sistema nervioso central (SNC), más la influencia de factores sobreañadidos (O2 y VM, entre otros).Las variables más predictivas fueron la ecografía craneal y la edad gestacional (AU)

Objective: To study the neurological evolution of very low birth weight newborns (VLBWN) (< 1.500 g) in our area. Patients and methods: Retrospective study of the neurological evolution of 268 VLBWN during a period of 5 years in a multidisciplinary approach with the participation of neonatology, pediatric neurology and psychology units. We have done periodical ophthalmologic explorations, electroencephalograms, and auditory evoked potentials. Period of the study: 1993-1999. The statistical study was done with SPSS and Windows. The damage were classified as mild, moderate and severe and according to the type of deficiency in motor, cognitive, sensorial, combined and plurideficiencies (when one or more lesions were present). The prevalence and mortality of VLBWN were also analyzed. Results: It is remarkable a mean weight low for gestational age, maternal age higher than 30 years of age, high percentage of low weight, cesarean sections, obstetric diseases, neurological and extraneurological complications, medical treatments and mechanical ventilation; low percentage of maternal treatment with corticoids and /or uterus relaxant medications and of premature newborns with suphactant. Assisted reproduction: 17,68%. Number of fetus > 1:37%. Te neonatal mortality rate of VLBWN has decreased along the years being 17,05% in 2005, 14,45% in 2006, and 11,18% in 2007. The incidence has increased being 18/1.000 in 2005, 18,3/1.000 in 2006, and 21/1.000 in 2007. Physcial damage was observed in 30%; mild in 46,5%, moderate in 22,1%, severe in 31,4%. The lesions were predominantly of motor type (43%), followed by combined in 27,9% and plurideficiencies in 17,4%. Neurosensorial deafness in 4,8%. Premature retinopathy in 17,2%. Neurosensorial deafness in 4,8%. Premature retinopathy in 17,2%. Cerebral palsy in 17,6%. 40% had some kind of alterations in the transfontanelle echography, 22,7% had intra/periventricular hemorrhage, 15.3% periventricular leukomalcia and 33% of the school age children had learning disabilities. The sequelae had high statistical correlation with membrane hyaline disease, bronchopulmonary dysplasia, late sepsis, oxygen dependency and mechanical ventilation, and specially with gestational age and transfontanelle echography alterations. Conclusions: We have observed an increment in the frequency and survival of the VLBWN. The incidence of damage is similar to other studies with a high percentage of severe deficiencies. The neurologic damage is mainly associated with gestational age and with central nervous system injuries; the influence of other factors such as oxygen and mechanical ventilation are also important. The most predictive variables are gestational age and transfontanelle echography (AU)

[Benign paroxysmal vertigo. Our 14 years' experience with this entity]. / Vértigo paroxístico benigno. Nuestra experiencia de 14 años.

INTRODUCTION: Benign paroxysmal vertigo (BPV) is characterised by the sudden onset of brief recurring episodes of loss of balance, sometimes accompanied by vegetative symptoms, which tend to disappear spontaneously in a matter of months or years and have no intercritical alterations. PATIENTS AND METHODS: We analyse the clinical and developmental characteristics of the patients who were listed as possible cases of benign paroxysmal vertigo in the database of the Neuropaediatric Unit of the Hospital Miguel Servet in Zaragoza over a 14 year period. Clinical records were reviewed and cases no longer controlled were contacted by telephone. RESULTS: 18 patients were considered to be cases of BPV, with the typical criteria, and 13 were found to be possible BPV (p-BPV). The atypical data of the p-BPV included late age of onset, prolonged persistence of the episodes and long duration of each episode. Neuroimaging was performed in 8 (44%) of the BPV and 10 (77%) of the p-BPV, and electroencephalogram recordings were carried out in 15 (83%) of the BPV and 11 (85%) of the p-BPV, with normal results in all cases. CONCLUSIONS: Establishing a diagnosis of BPV with strict criteria can mean that some atypical cases are excluded. As no biological marker is available, the diagnosis is clinical-developmental and sometimes complementary tests need to be conducted in order to rule out other pathological conditions. A broader clinical spectrum, cases in which the episodes last longer and a greater range of ages of onset and complete remission are all possible. A diagnosis of BPV should reassure the patients' families as it is a benign process that tends to disappear spontaneously.

Vértigo paroxístico benigno. Nuestra experiencia de 14 años / Benign paroxysmal vertigo. Our 14 years’experience with this entity

Introducción. El vértigo paroxístico benigno (VPB) se caracteriza por episodios breves y recurrentes de pérdida de equilibrio, de inicio brusco, acompañados en ocasiones de síntomas vegetativos, con tendencia a la desaparición espontánea en meses o años y sin alteraciones intercríticas. Pacientes y métodos. Se han analizado las características clínicas y evolutivas de los pacientes que figuran como posibles casos de vértigo paroxístico benigno en la base de datos de la Sección de Neuropediatría del Hospital Miguel Servet de Zaragoza durante un período de 14 años. Se revisaron las historias y se contactó telefónicamente con los casos que no seguían control. Resultados. Se han considerado 18 pacientes VPB, con los criterios típicos, y 13 como posible VPB (p-VPB). Los p- VPB tuvieron como datos atípicos edad tardía de presentación, persistencia prolongada de los episodios y duración larga de los episodios. Se realizó, con resultados normales, neuroimagen a ocho (44%) de los VPB y a diez (77%) de los p-VPB, y electroencefalograma a 15 (83%) de los VPB y a 11 (85%) de los p-VPB. Conclusiones. Establecer el diagnóstico de VPB con criterios estrictos puede excluir algunos casos atípicos. Al no disponer de un marcador biológico, el diagnóstico es clinicoevolutivo y, en ocasiones, deben realizarse exámenes complementarios para descartar otras patologías. Puede haber un espectro clínico más amplio, casos de mayor duración de los episodios y mayor rango en las edades de presentación y desaparición. El diagnóstico de VPB debe tranquilizar a las familias, al ser un proceso benigno con tendencia a la desaparición espontánea

Introduction. Benign paroxysmal vertigo (BPV) is characterised by the sudden onset of brief recurring episodes of loss of balance, sometimes accompanied by vegetative symptoms, which tend to disappear spontaneously in a matter of months or years and have no intercritical alterations. Patients and methods. We analyse the clinical and developmental characteristics of the patients who were listed as possible cases of benign paroxysmal vertigo in the database of the Neuropaediatric Unit of the Hospital Miguel Servet in Zaragoza over a 14 year period. Clinical records were reviewed and cases no longer controlled were contacted by telephone. Results. 18 patients were considered to be cases of BPV, with the typical criteria, and 13 were found to be possible BPV (p-BPV). The atypical data of the p-BPV included late age of onset, prolonged persistence of the episodes and long duration of each episode. Neuroimaging was performed in 8 (44%) of the BPV and 10 (77%) of the p-BPV, and electroencephalogram recordings were carried out in 15 (83%) of the BPV and 11 (85%) of the p-BPV, with normal results in all cases. Conclusions. Establishing a diagnosis of BPV with strict criteria can mean that some atypical cases are excluded. As no biological marker is available, the diagnosis is clinical-developmental and sometimes complementary tests need to be conducted in order to rule out other pathological conditions. A broader clinical spectrum, cases in which the episodes last longer and a greater range of ages of onset and complete remission are all possible. A diagnosis of BPV should reassure the patients’ families as it is a benign process that tends to disappear spontaneously

[Absence epilepsy. A review of our 14 years' experience]. / Epilepsia con ausencias. Revisión de nuestra experiencia de 14 años.

INTRODUCTION: Absence epilepsy (AE), typically occurring at the paediatric age, is characterised by episodes of diminished consciousness accompanied by a generalised rapid spike-wave in electroencephalogram recordings. PATIENTS AND METHODS: Our study involved children with AE from the Neuropaediatrics database between May 1990 and May 2004. Patient records were reviewed and cases no longer controlled were contacted by telephone. RESULTS: Of a total of 7,562 patients surveyed in the period under study, 757 subjects (10%) had epilepsy and there were 49 cases of AE (6.47% of the total number of cases of epilepsy): 29 were females (59.2%) and 20 were males (40.8%). Mean age at the time of the first visit was 7.93 years (ranging between 3 years and 10 months and 13 years and 6 months). The average follow-up time between the first visit and the last time information was updated was 5.3 years (ranging between 10 days and 13 years and 2 months). Only two females, receiving treatment, still have absences. 42 children have been without absences for more than six months, 16 with treatment and 26 without therapy; 21 children have been without absences for over four years and are not under treatment. 12 have problems at school. CONCLUSIONS: AE is easy to diagnose and usually responds well to treatment either as monotherapy or, in some cases, in association with two antiepileptic agents. Strict initial control by experts in its management prevents absences from continuing over long periods of time. The psychosocial and learning dysfunctions that are associated in some cases require close attention.

Epilepsia con ausencias. Revisión de nuestra experiencia de 14 años / Absence epilepsy. A review of our 14 years experience

Introducción. La epilepsia con ausencias (EA), propia de las edades pediátricas, se caracteriza por episodios de disminución de la conciencia acompañados de punta onda rápida generalizada en el electroencefalograma. Pacientes y métodos. Niños con EA de la base de datos de Neuropediatría desde mayo de 1990 hasta mayo de 2004. Se han revisado las historias clínicas y se ha contactado telefónicamente con los casos que ya no se controlaban. Resultados. De 7.562 pacientes valorados en el período de estudio, figuran 757 pacientes (10%) con epilepsia y 49 casos de EA (6,47% del total de las epilepsias): 29 mujeres (59,2%) y 20 varones (40,8%). La edad media en el momento de la primera consulta era de 7,93 años (rango entre 3 años y 10 meses y 13 años y 6 meses). El tiempo medio de seguimiento entre la primera visita y la última actualización de datos es de 5,3 años (rango entre 10 días y 13 años y 2 meses). Sólo persisten ausencias en dos niñas, en tratamiento. Hay 42 niños que llevan más de seis meses sin ausencias, 16 con tratamiento y 26 sin tratamiento; 21 niños llevan más de cuatro años sin ausencias y no están con tratamiento. 12 niños presentan dificultades escolares. Conclusiones. La EA es de fácil diagnóstico y, habitualmente, buena respuesta terapéutica, con monoterapia o, en algunos casos, la asociación de dos antiepilépticos. Un estrecho control inicial por expertos en su manejo evita la persistencia de las ausencias durante períodos prolongados. Deben vigilarse disfunciones psicosociales y de aprendizaje que se asocian en algunos casos (AU)

Introduction. Absence epilepsy (AE), typically occurring at the paediatric age, is characterised by episodes of diminished consciousness accompanied by a generalised rapid spike-wave in electroencephalogram recordings. Patients and methods. Our study involved children with AE from the Neuropaediatrics database between May 1990 and May 2004. Patient records were reviewed and cases no longer controlled were contacted by telephone. Results. Of a total of 7,562 patients surveyed in the period under study, 757 subjects (10%) had epilepsy and there were 49 cases of AE (6.47% of the total number of cases of epilepsy): 29 were females (59.2%) and 20 were males (40.8%). Mean age at the time of the first visit was 7.93 years (ranging between 3 years and 10 months and 13 years and 6 months). The average follow-up time between the first visit and the last time information was updated was 5.3 years (ranging between 10 days and 13 years and 2 months). Only two females, receiving treatment, still have absences. 42 children have been without absences for more than six months, 16 with treatment and 26 without therapy; 21 children have been without absences for over four years and are not under treatment. 12 have problems at school. Conclusions. AE is easy to diagnose and usually responds well to treatment either as monotherapy or, in some cases, in association with two antiepileptic agents. Strict initial control by experts in its management prevents absences from continuing over long periods of time. The psychosocial and learning dysfunctions that are associated in some cases require close attention (AU)

[Cleft palate and cleft lip. Clinical review]. / Fisura palatina y labio leporino. Revisión clínica.

UNLABELLED: The aim of this study is to do an analytical study of cleft palate and cleft lip in our hospital. PATIENTS AND METHODS: 85 clinical charts of patients attended in our hospital born between 1976 and 2001 in Aragon and Rioja were reviewed. We studied the incidence of oral cleft, associated malformations and morbidity, familial antecedents and perinatal data, phonatory disfunctions, serose otitis, growth failure and psychiatry problems. RESULTS: The mean incidence was 0.5/1000 newborns. 41.5% presented associated malformations and 19.3% were associated with a specific syndrome, being more frequent in patients affected of cleft palate and cleft lip (50%) than patients with only cleft palate (41.2%) or only cleft lip (8.8%). The most frequent malformations were: facial defects (50%), skeletal (33%), congenital cardiopathies (33%). 19% were born prematurely. The percentage of serose otitis that required control at hospital was 37.3%. 34.2% presented phonatory problems. There was a high incidence of growth failure and psychiatry problems. CONCLUSION: Oral clefts represent a complex clinical condition with a high percentage of medical complications that require a multidisciplinary treatment. The high incidence of congenital defects associated with this condition demand an exhaustive screening in the newborns affected.